While the title of this piece implies there are both harms and benefits, I have not been able to find any benefits of this new DSM V diagnosis. Many health professionals are already warning about the harm it will cause – especially to people with devastating physical symptoms of an as-yet-undiagnosed illness.

There are five possible explanations whenever someone presents to a doctor with physical symptoms that have not yet been diagnosed:

Like this:

Like any fourteen-year-old preparing for high school, Emily Lemiska felt self-conscious about her appearance. She wasn’t worried about her weight, hair or skin. Emily was self-conscious about her abnormally short neck. She asked her parents to make an appointment with her pediatrician to take a look. Emily, her parents and her doctor alike were shocked when an X-ray showed she had Klippel-Feil syndrome (KFS).

Klippel-Feil is a spine disorder characterized by the fusion of two or more cervical vertebrae, which decreases range of motion and flexibility in the neck. It is known to cause pain, especially later in life, and increases the dangers of even minor trauma to the neck. With reports estimating the condition occurs in one in 40,000 live births, KFS is considered a rare disease. Emily’s case is even more atypical in that seven of her vertebras, C2-T1, are fused.

Fortunately, Emily was asymptomatic, with no pain or discomfort. Nor did she appear to have any of the additional abnormalities – ranging from heart defects to hearing loss – sometimes associated with KFS. Although she could no longer participate in some of her favorite activities like playing volleyball or riding rollercoasters, which put her at risk of whiplash or other injuries, she was able to maintain a normal life. While doctors continued to monitor her neck annually, her health thankfully stayed the same. Although she felt a little isolated because of her condition, for the most part, instead of worrying about KFS, Emily was able to worry about the usual teenage woes like boys and grades.

Determined to experience life to the fullest, Emily left her small town in Connecticut to attend Northeastern University in Boston. She excelled in her classes, formed friendships with a tight-knit group of honors students, and met her now-husband, Dan. She was extremely active in extracurricular activities, serving as editor-in-chief of the literary arts magazine, vice chair of student media and copy editor at the newspaper. Even with her busy schedule at school, she managed to work part-time and volunteer on a regular basis.

In 2008, after a semester abroad in Barcelona, Emily graduated summa cum laude with a bachelor’s degree in English. She accepted a position at Mass General Hospital in the public affairs department, where she served as editor of the hospital-wide newsletter, spearheaded communications campaigns and interacted with local media. Her job was chaotic at times, but she loved it. She took pride in her work and became a valuable asset to the team.

Emily found an outlet from her demanding job in the form of running and weightlifting. She liked the way physical activity made her feel, and the doctors who continued to monitor her encouraged an active lifestyle. She had no idea that an upcoming five-mile run would change the course of her entire life.

That springtime “fun run” around the Charles River in 2011 would be the last time she ever ran. The day after the race, Emily was startled by brutal shoulder and neck spasms that crippled her with pain. When they didn’t subside in a few days, she made an appointment to see her doctor. He was perplexed. X-rays and MRIs didn’t reveal any reason for her sudden symptoms. He assumed she strained a few muscles, prescribed Valium and a neck brace, and suggested taking it easy for a few weeks.

The next two months were torture. Emily couldn’t use her arms or lift anything without excruciating muscle pain; even typing at work irritated her shoulder muscles. With every movement, her entire spine felt as though it was being yanked. Walking and riding the bus to and from work became dreaded endeavors, and any vibration caused unimaginable discomfort. Getting through the workday became her sole focus: she quit exercising, stopped volunteering at the local library and declined invitations from friends. Dan and her two roommates had to help her with even the smallest tasks, like making dinner and cleaning.

The symptoms only intensified. In July, Emily woke up before dawn to discover her left side completely numb. Terrified, she called her parents and then took a taxi to the emergency room. Again, the doctors were at a loss.

The ER visit led to a consult with a neurosurgeon. Within minutes of reviewing her neck imaging, the physician told Emily and her dad that she had another abnormality besides fused vertebrae: a tethered spinal cord. This neurological disorder is caused when spinal cord tissue attaches to the spinal column, limiting the movement of the spinal cord. He also discovered that in the same area, Emily had diastematomyelia or a split spinal cord. In hopes of halting the progression of these conditions and lessening the pain, he proposed emergency neurosurgery to untether her spinal cord.

Dan’s commitment never wavered despite the stress on their relationship. In fact, he embraced Emily more fully than ever, proposing to her three days prior to her operation. In the days leading up to surgery, Dan began referring to her as his “brave little toaster,” a reference to the 1987 Disney movie about an animated toaster who faces many obstacles on his journey to find his owner.

The six-hour surgery, by technical standards, was a success, and the spinal cord was freed from the spinal column. After seven days in the ICU, she went home to Connecticut to finish her recovery. Determined to return to normal life, Emily went back to work only a month and a half later. She immediately realized she had returned too soon. The pain returned quickly and with vengeance.

For the next year and a half, Emily put on a brave front as she tried to keep the life she loved. Work was excruciating – she would sneak off to lie down in the conference room, come in late and leave early, and work from home as much as possible.

“I was absolutely miserable, but too stubborn to show it outright. By the time I got home at night all I had the energy to do was cry. I felt completely dehumanized by pain.”

Weekends were no longer spent enjoying all that Boston had to offer. Instead, she would lie in bed, trying to recover from the week before and prepare for the one ahead. All the while, the muscle spasms and nerve pain were unrelenting.

Her frequent doctor visits left her discouraged as well. While following the doctors’ suggestions, nothing subdued the pain. Because her muscles were irritated and her spinal cord increasingly sensitive post-surgery, treatments like physical therapy and injections would sometimes even exacerbate her issues.

In December 2012, Emily made the difficult decision to leave her position at Mass General Hospital. Much of how she defined herself was her successful career. Quitting was a huge loss, but she had no choice. With Klippel-Feil being a degenerative syndrome, Emily had to slow down. She needed to change tactics, and instead of constantly playing defense against her aggressive symptoms, she had to go on the offense. It was important for her to protect her baseline so as to not regress further.

Back in Connecticut, Emily and Dan moved in with her parents for six months before finding an apartment nearby. Emily’s dad now drives her wherever she needs to go, and her mom, a registered nurse, attends all of her major doctor’s appointments. This extra help has been much appreciated – knowing that Emily would not be able to work, Dan is attending law school in hopes that his career might make enough income for two.

To manage the pain, Emily takes more than 10 pills a day. Eager to be free from the side effects of her medications – including fatigue and mental cloudiness – Emily continues to pursue treatments that don’t come in tablet form. She and Dan also hope to someday start a family, and the drugs she takes are not conducive to pregnancy. Among the options she is considering is a spinal fusion surgery. This would entail implanting rods and screws to reinforce her spine. Although it might be her best option, there are great risks involved, and doctors are not sure whether it will help significantly. The rarity of her case means it is impossible to know whether it’s the right decision – there is nothing to compare to, no KFS studies to point to a positive outcome.

If she does choose to have surgery, Emily knows that it may not be a full solution. She hopes that the right combination of Western medicine and complementary therapies might bring relief. An epidural nerve block, for example, decreased nerve pain in her face for a short time. Acupuncture and massage also help with the pain, as does wearing a neck brace and heat and ice therapy. To keep the rest of her body active, Emily stretches every day, goes for short walks several times a week and recently began swimming. All of these activities require modifications; for example, she swims using a snorkel mask to avoid having to move her neck to breathe. But Emily says that doing an adapted version is far better than doing nothing at all. The goal of being a mom and publishing her personal writing one day drives her to stay positive and proactive during her search for better answers.

Emily’s life has completely changed due to the progression of her disease. She has had to redefine herself entirely. Not being able to work, having put such emphasis on her job, has been a major loss. She misses her hobbies, like running, playing the piano, cooking and volunteering. It especially bothers her that she can no longer help others, but instead, is the one who constantly needs help.

“It is hard to be 28 and unable to enjoy life as much as I want to,” she says. “My to-do list and my body don’t see eye-to-eye. Each day I have to find a balance between pushing myself enough to feel accomplished, but not so much that I’m hurting myself.”

Emily still does the things she loves, but in small doses with lots of rest in between. And even though her activity is limited, she says she never feels bored. To keep busy, she reads, listens to podcasts and TED talks, takes online courses, meditates and writes. She continues to do occasional writing projects as a freelancer, but only as the pain allows. She also enjoys taking trips with Dan and having friends over to visit. Emily jokes that even with all she does, it’s difficult not to feel like a professional sick person. Much of her daily routine consists of taking care of herself, scheduling appointments and dealing with insurance and disability paperwork.

While initially denied disability earlier this year, thanks to help from a state health care advocate, she was approved in September after a long appeal process. The stress of being disbelieved and misjudged was difficult to take. Knowing there is a negative public view of those needing to use disability benefits, she wishes others understood that the majority applying for help really need it. Like her, they want to work but are truly unable to due to severe health limitations. Without assistance, she is incapable of supporting herself. In fact, she and Dan had to rely on food stamps for a few months just to get by.

Emily is not ashamed talking about her personal trials, even financial ones. In fact, she is very open about the truth many in the pain community experience every day, even when it is hard for others to hear. She feels if more people were open about their hardships, there would be fewer stigmas and less misunderstanding about chronic pain. She believes sharing struggles does not make a person weak or vulnerable, but shows strength.

For this reason, Emily keeps a blog. Not only is it personally cathartic for her, but it is also a way for family and friends to stay in the loop. Occasionally, posts are so widely shared that they serve to help increase awareness about chronic pain among individuals outside her inner circle.

Emily believes all who live with pain should keep some sort of blog. It is a way to express emotions that might otherwise be difficult to release, while allowing those who know you a chance to understand more about your challenges.

Through her experiences, Emily realizes that giving up is not an option. If she could stress one thing to her pain peers it would be to become an expert on their particular diseases. “You have to advocate and fight for your care. Answers may be difficult to find, but never stop searching or hoping. It may take time, but the medical community makes advances every day. You don’t want to be the one to give up the day before they find the treatment the helps you.”

Emily keeps abreast of developments in spine care through Google Alerts and by reading research abstracts from medical journals. She stays on top of her own care by requesting and reading her medical records, bringing a list of questions and taking notes during appointments, and getting multiple second opinions when necessary.

Emily also stays connected and informed through the resources she receives from the Klippel-Feil Syndrome Freedom. This small, grassroots nonprofit is trying to help people afflicted with the disease obtain support, strength and information. Created by other Klippel-Feil patients, the organization is personally dedicated to the cause.

Through this group, Emily finally met another individual with this disease, fourteen years after her diagnosis. Being able to connect with someone like her was life changing. For the first time, Emily did not feel so isolated and alone in the world. She had met someone who fully understood – and she was delighted to see that this fellow patient had two children of her own.

In her small way, she is doing her part to advance care for KFS patients. She is working on a KFS survey to collect data on patterns of abnormalities, symptoms and treatments tried. She hopes the results will help inform the medical community while empowering those living with the devastating disease. She is also planning a holiday fundraiser for 2015 – featuring a skeleton key holiday ornament – with proceeds benefiting KFS Freedom.

Cheerful and determined, Emily chooses to live in gratitude. While Klippel-Feil is progressive, she knows she is blessed with an amazing support system. Her parents, friends and husband go above and beyond to show her she is loved and that she is never alone. Her doctors aren’t sure how much worse her condition might become as time passes. But Emily is not giving up on life; rather, she is embracing it.

“People often seem surprised at how positive I am,” says Emily. “We all have a tendency to underestimate ourselves. No matter what life throws at you, you can and will find a way to live the best life possible.”

What Are Trigger Points

In simple terms, a trigger point is a knot that forms in the muscle and sends pain to other areas of the body. Trigger points cause the muscle to become tighter and shorten. When muscles shorten, they cannot go through the full range of motion, altering the way you move, sit or stand. This leads to strength and flexibility issues, creating more trigger points.

Research suggests that fibromyalgia pain is largely due to myofascial trigger points. Therefore, treatment of trigger points will help manage the pain associated with fibromyalgia.

Trigger Points In Neck That Cause Dizziness

The trigger points in the neck that can cause dizziness form in the sternocleidomastoid (SCM) muscles. The SCM is a large muscle along the front on both sides to the neck. It is made up of two interconnected muscle bands. These muscle bands start out from the mastoid bone behind the ear. One band connects to the breastbone (sternum) and the other connects to the collarbone (clavicle). The sternal band lies on top of the clavicle band.

The primary functions of the SCM muscles are to turn the head from side to side and flex the head downward. The sternocleidomastoids also help maintain a stable position of the head during other body movements. Any position where the neck is held in an awkward position can create trigger points.

Another function of the SCM muscle is to raise the breastbone when you inhale. The muscle can become overworked if you breathe with the chest, rather than with the diaphragm. The SCM also assists with chewing and swallowing.

Symptoms Of Sternocleidomastoid Trigger Points

The effects of sternocleidomastoid trigger points can be amazingly widespread. Symptoms created by SCM trigger points include:

Trigger points can be created by postures that keep the SCM contracted to hold the head in position -for example, looking at a computer screen or driving. Keeping your head turned to one side or holding your head back to look up for extended periods of time, are sure to cause problems. Breathing from the chest instead of the belly can also overwork the SCM muscle.

Here is a list of activities that might create SCM trigger points:

Overhead activities

Keeping your head turned to one side

Forward head posture

Holding phone with shoulder

Stomach sleeping

Heavy lifting

Falls and whiplash

A short leg or scoliosis or awkward posture

Stress and muscle tension

Chronic cough or asthma

Chest breathing

Sternocleidomastoid Trigger Point Release

SCM trigger points are easily self-treated. The SCM muscle group can contain seven trigger points. The sternal division typically has 3-4 trigger points spaced out along its length, while the clavicle division has 2-3 trigger points.

NEVER massage a pulse. If you pinch the sternocleidomastoid, rather than press it against the side of the neck, you will stay off the arteries.

Follow these steps to release the SCM trigger points:

While looking in a mirror turn your head to one side. You will see the sternal branch.

Grasp the muscle with your thumb and fingers curled into a C shape and turn your head back to face the mirror.

Keeping your face looking forward, tilt your head slightly down and to the same side you are massaging.

Press only hard enough that it feel comfortable and try to discriminate between the two branches. Each branch is about as big as your index finger. If you pay close attention, you should be able to feel them separately.

Milk the muscle with short repeated up and down strokes, start in the middle and work your way up to behind your ear and then all the way down to the collarbone.

If you find a spot that hurts, gently pinch the trigger point. Reduce the pressure until you don’t feel any pain. Once you’re below the pain threshold, slowly increase the pressure over 60-90 seconds.

Do this on both sides, a couple of times a day. Just go easy at first, and work at a pressure level that feels good for you. For a visual demonstration, you can watch the video and learn more at the original post, linked below.

What follows is a truly fascinating look at why so many Ehler-Danlos Syndrome patients (especially Hypermobility Type, also used to be called EDS-III or Type 3), including myself, languish in pain, not taken seriously, waiting for a correct diagnosis. I accurately fit every requirement for EDS and was born with bilateral hip dislocations, a hole in my heart, and Spina Bifida Occulta affecting both my lower spine in a visible dimple and then at the area where the disc C2-3 should be I have instead two fused vertebrae not caused by injury or surgery. Those same physical therapists and radiologists have told me that I have craniocervical instability, but the PT couldn’t do much about it except help me find exercises that were somewhat safe for my neck.

In a slow car accident involving a semi truck with three trailers hitting my car on my way to work, I sustained two fully torn vertebral discs, and at least four other bulging/slipped discs. That much damage from a car accident that didn’t even total my used vehicle? Totally a give-away for Ehler Danlos Syndrome. I know that Spina Bifida is somewhat more common in EDS families, but I don’t know if there has every been an official link acknowledged between the two, although being born C-section with dislocated hips should be a pretty good indication that I had faulty joints. It’s crazy that my doctors continue to ignore my pleas for a solid EDS diagnosis even though I fulfill the Beighton and Brighton scales/scores on every move, and even though as a child I was known in my gym as “rubberband girl”. That was in comparison to all the other ages of girls there too, some of whom competed and did very well, but were never as flexible as me. I injured myself too many times and healed too slowly to keep it up into puberty . Isn’t that almost the same story of every person with EDS who participated in rigorous and physically demanding sports not knowing they had a collagen problem?

Further proof comes from my mom’s knee cartilage disintegrating in one night of dancing, according to her, and never being the same afterwards. Also the way we scar, and the hormonal imbalance most of us have. All the hip problems, bowel problems, arthritis and vascular issues that run in my family? Probably tied in to EDS genes, is my best guess, and those are all definitely areas of the body affected by collagen or lack thereof. All areas of the body are affected by collagen production. The craziest part is that I have instead been called a liar, been misdiagnosed several times and then called a liar again, which I pushed through only to be assigned many of these so called “wastebasket diseases” for which there is no real standard of care that works for all or even most patients. I know, horrible, horrible name, “wastebasket disease” and it sucks to be in that category because many doctors actually treat you like trash. At the best they mean well but have no idea how to help you significantly.

I don’t know why I have been diagnosed with JHS since my birth, back when it was known as Benign Hypermobility Syndrome (benign, my ass), but in texts now JHS and EDS-HT are medically acknowledged to be the same disease with the exact same treatment recommendations except that with the diagnosis of EDS my doctors may understand why tiny doses of opiates have never and will never cut it. I’m so opposed to any kind of surgery until they understand if I require more anesthetic during surgery than a non-EDS patient.

When I was young and injured myself pretty much once a month, doctors would look at my bones on x-ray film and say that they looked like the bones of a much older person but that I should be fine because I have bigger bones and that should help protect me. I’m not fine, doctors! Help!!! Send me a time machine or at least a geneticist who will take me seriously! I have already lost so much mobility and flexibility, and my spine is so harshly curved now in two places that it is starting to be difficult to get dressed, my fingers get stiff and spasm a lot more, as well as dislocate with the slightest of tasks, even typing. It’s not super painful unless they dislocate in a specific way. There are places it’s happened so many times I don’t notice it except when the joints get stuck and won’t move, like my knees for instance!

The studies that have been done recently say that 90% or more of all EDS sufferers have no idea what is wrong with them, or they know but can’t see a geneticist to confirm, due to lack of clear diagnostic criteria and no clearly defined specialists who commonly deal with the genetic condition. Then there is the often prohibitive cost of genetic testing. I can see why so many of us get left to rot. And there are probably a good deal of high functioning EDSers out there who weren’t dancers or gymnasts and who didn’t abuse their bodies as much as I did, and their life will likely be normal enough that if they learn of it, it will be because of having a child who has EDS, more than likely. I want to find out before that!!! That abundance of undiagnosed EDSers living with the disease seems backed up by all the patient populations they examined in the below article. The high occurrence of fibromyalgia alongside EDS-HT (around 50% of the fibro patients had EDS markers, and around half of the studied EDS population were found to have all key fibromyalgia symptoms) makes perfect sense as outlined by the last reblog I did from EDS Info. That post deals with the fact that Untreated Chronic Pain is a Medical Emergency, where chronic pain states are explained as often arising from untreated acute pain after trauma, which is totally true in my case. I was too young to be in real pain, because that’s a thing, and my car accident wasn’t impressive enough that I merited correct dosage of narcotics, and I was shamed into not asking for them as often as I needed them.

Anyhoo, rant aside, the article is an elegant, and unique, explanation of so much that is difficult about navigating in the world of chronic illnesses and differential diagnoses.

Ehlers-Danlos Syndrome Hypermobility Type: A Genetic Predisposition to the Development of Various Functional Somatic Syndromes

Introduction

Functional Somatic Syndromes, conditions characterized by functional disability and self reported symptoms rather than clearly demonstrable organic problems, are a common contemporary health issue [1]. Each medical subspecialty seems to have at least one somatic syndrome for patients whose symptoms cannot otherwise be medically explained. These include: irritable bowel syndrome (gastroenterology); fibromyalgia (rheumatology); tension headaches (neurology); and chronic fatigue syndrome (immunology) [2]. In recent years, however, a significant portion of these patients have gone on to receive a diagnoses of a little known connective tissue disorder: Ehlers-Danlos syndrome hypermobility type (EDS-HT), formerly type III [3]. In this literature review, I will discuss the features of EDS-HT, explore EDS-HT as a possible unifying concept for various functional somatic syndromes, illuminate further implications of the described findings, outline a set of diagnostic criteria that should be implemented by healthcare professionals in functional diagnostic medicine, and propose a novel way of thinking about functional somatic syndromes.

Ehlers-Danlos Hypermobility Type (EDS-HT) Overview

EDS-HT, considered to be one and the same with joint hypermobility syndrome (JHS), is a relatively common, frequently underdiagnosed heritable condition which predisposes those afflicted to chronic, widespread musculoskeletal pain and a wide variety of articular and extra-articular features purportedly linked to constitutionally abnormal collagen. The diagnosis is primarily clinical in essence and is largely based on the Beighton score (a simple system used to quantify joint laxity and hypermobility) and medical history. It is predominantly of autosomal dominant inheritance, though the molecular basis of EDS-HT is still largely unknown except for a minority of patients mutated in TNXB and COL3A1 [4]. Skin biopsies may show alterations in collagen fibril morphology [5]. Early literature fixed the frequency of EDS as a whole to 1 in 5000, with EDS-HT accounting for approximately half of all registered cases. However, due to it’s vast underdiagnosis, a presumed frequency of 0.75-2% has been proposed for EDS-HT [4].

Hypermobility and the Autonomic Nervous System:

The Missing Link for Various Functional Somatic Syndromes

When first described, EDS-HT was considered to be a relatively benign condition, with acute and chronic joint instabilities as it’s unique clinical consequence. Recently, however, accumulated experience on the management of EDS-HT patients elucidated a more complex clinical picture. In particular, subjects with joint hypermobility appear to be more prone to developing a range of functional somatic syndromes [3], including fibromyalgia [6], chronic fatigue syndrome [7], headaches [8], complex regional pain syndrome [10], gastrointestinal functional disorder [11], pelvic organ prolapse [12], and orthostatic intolerance [13].

An underlying dysautonomic process may explain many of the aforementioned functional somatic syndromes seen in EDS-HT individuals, which are present in practically all major body systems. Leading research suggests that the pathogenic relationship between dysautonomia and congenital laxity of the connective tissue is primarily attributable to the pathological deformation of the brainstem and upper spinal cord from occipitoatlantoaxial hypermobility and cranial settling [8]. In other words, craniocervical hypermobility and instability, and the resulting deformative stress of repetitive stretching and ventral brainstem compression, appear to underlie the observed autonomic dysfunction in hypermobile patients [9]. As demonstrated in pathological reports of fatal cases of traumatic brain injury and numerous animal studies, repetitive stretching of nerves can lead to clumping and loss of neurofilaments and microtubules within the axon and promotes neural apoptosis [14][15]. Strain also alters the electrochemistry of the nerve by decreasing the amplitude of action potentials [16] and increasing calcium influx into the cell [17]. When you apply this research to the context of hypermobile individuals, the underlying process of autonomic nervous system dysfunction becomes palpable. Unsurprisingly, the histopathological changes in neural axons that are undergone in these situations would not show up on any routine diagnostic test. In extreme cases, however, cranial settling and a reduction of the clivo-axial angle may be demonstrable on MRIs, but typically only when imaged in the upright position [8]. This would explain why many of these patients’ diagnostic imaging reports state negative results.

In accord with craniocervical hypermobility findings, recent studies have suggested that up to 70% of patients with hypermobility have orthostatic intolerance and other forms of dysautonomia. The orthostatic effect in EDS-HT individuals may also be compounded by abnormal connective tissue in the vasculature, which results in an increase in blood vessel distensibility in response to the augmented hydrostatic pressure that occurs during orthostatic stress. This leads to exaggerated blood pooling in the lower extremities with a resultant tachycardia [18]. While these findings were predictable, a reversed frequency study, wherein hypermobility was measured in patients diagnosed with Postural Orthostatic Tachycardia Syndrome, a prevalent form of dysautonomia in young people, found that an extraordinary 53% of participants met the diagnostic criteria for EDS-HT [19]. Furthermore, when hypermobility was measured in individuals diagnosed with Chronic Fatigue Syndrome, a condition with a longstanding, established association with orthostatic intolerance [20], researchers found that 25% of Chronic Fatigue syndrome sufferers had generalized hypermobility [21]. This phenomena, though, is likely of multifactorial consequence, as dysautonomia, chronic pain, and sleep apnea secondary to ventral brainstem compression can result in poor sleep architecture and chronic fatigue [22][23][24].

Ehlers-Danlos Syndrome Hypermobility Type as a Systemic Condition

The autonomic nervous system problems associated with hypermobility, alike various functional somatic disorders, are present in practically all major body systems. In the realm of gastroenterology, for instance, dysautonomia in the form of vagus nerve damage (which may result from craniocervical instability) can result in delayed gastric emptying [25] and affect bowel contractibility, causing nausea and the so called “irritable bowl syndrome” [26]. Moreover, the underlying collagen abnormality of EDS-HT itself is systemic. Insufficient collagen may reduce sphincter tone and increase distensibility of the gut wall (which is likely to influence the function of surrounding cellular mechano-receptors), resulting in decreased gastrointestinal motility, gastroesophageal reflux (GERD) and/or irritable bowel syndrome (IBS). In fact, over 50% of EDS-HT individuals have GERD and/or IBS [4][27]. When hypermobility was tested in patients diagnosed with functional gastrointestinal disorders (which include IBS, functional dyspepsia, and functional constipation), an astonishing 49% were found to have joint hypermobility and many of those patients went on to receive an official diagnosis of EDS-HT [10].

When it comes to neurological manifestations, headaches are among the most common complaint in the EDS-HT population [4]. As a consequence of occipitoatlantoaxial hypermobility, drooping of the cerebellar tonsils and obstruction of the cerebrospinal flow at the craniocervical junction can result in intracranial pressure [8][28]. In addition, rapid fluctuations in blood pressure and inadequate cerebral perfusion on upright posture caused by dysautonomia may lead to migraines [29][30]. People with lax joints are also predisposed to cervicogenic, tension, and new daily persistent headaches arising from musculoskeletal dysfunction in the temporal mandibular joints and the upper three cervical segments of the spine [4][31].

As a consequence of ligamentous laxity, rheumatological complications among the EDS-HT population are commonplace. Chronic pain in patients with joint hypermobility stems from a predisposition to injury from daily minor trauma to the joints and ligaments [32]. Unstable joints may also lead to frequent dislocations, subluxations, sprains, and stretch injury to the nerves traversing hypermobile joints, further increasing the risk of developing chronic pain states such as arthralgia, repetitive strain injuries, and complex regional pain syndrome [4][9][33]. There is also a high incidence of muscular pain attributable to myofacial spasms. Tender points consistent with fibromyalgia are often palpable, especially in the paravertebral musculature [34]. In frequency studies, the prevalence of fibromyalgia in EDS-HT participants was established to be 30% [35] and the prevalence of EDS-HT among fibromyalgia subjects was found to be 27.3% [6]. One theory for the origin of pain in fibromyalgia ascribes it to excessive muscle stress, which may increase the excitability of nociceptive ends of the muscle [36][37]. Joint instability in hyperlax individuals may result in sustained muscle stress (an overcompensation mechanism for loose and injured joints) and over stimulation of nociceptive nerve endings (which are poorly supported by defective collagen fibrils) [38]. An alternative, although equivocal, theory has suggested that biomechanical disturbances in the cervical spine may play a role in the pathogenesis of fibromyalgia. In a controlled study of 161 cases of traumatic injury to the cervical spine (primarily “whiplash”), fibromyalgia was diagnosed in 21.6% of those with neck injury verses 1.7% control subjects with lower extremity fractures [39], bringing us back to the notion that craniocervical instability, and the subsequent neurological damage, may be the underlying process in the development of functional somatic syndromes.

Further Implications of Discussed Findings in the Diagnosis and Management of Functional Somatic Syndromes

These observations suggest that a careful examination for hypermobility and connective tissue abnormalities should be an integral part of functional diagnostic medicine. Pathological deformation of the brainstem and stretch injury to neural axons due to an underlying congenital ligamentous laxity, as discussed here in the case of EDS-HT, or acquired ligamentous instability, such as whiplash, may indeed be the missing link in the pathogenesis of various functional somatic syndromes.

In a literature review of functional somatic syndromes, Wessely and colleges concluded, “a substantial overlap exists between the individual syndromes and that the similarities between them outweigh the differences” and “patients with one syndrome frequently meet diagnostic criteria for another” [40]. For this subset of patients, generalized joint hypermobility may represent the common milieu for functional somatic syndromes with ubiquitous manifestations. The predispositions EDS-HT imposes would further explain why many of these patients are affected profoundly by emotional arousal (as it’s mediated by the autonomic nervous system) and muscle tension, and why patients with different syndromes share non-symptom characteristics such as sex (as joint laxity is more pronounced in females) and develops at a relatively young age (as EDS-HT is heritable, and hence, lifelong) [4][41].

Accordingly, articular hypermobility can be assessed by using the 9-point Beighton score, which assigns one point for each side of the body on which the patient can (1) passively dorsiflex the 5th finger >90 degrees with the forearm flat on the table, (2) passively appose the thumb to the flexor aspect of the forearm, (3) hyperextend the elbow beyond 10 degrees, and (4) hyperextend the knee beyond 10 degrees and one point for forward flexion of the trunk with the legs straight so that the palms rest flat on the floor. If a patient receives a Beighton score of 4 or more, a referral to a geneticist or rheumatologist for further evaluation is recommended [42]. If cranial settling and a reduction in the clivo-axial angle is suspected, and upright MRI may additionally aid in diagnosis [8].

With this hitherto unobserved connection comes a new line of treatment for a subdivision of patients with functional somatic disorders. Physical therapy, in the form of exercises that strengthen joint-supporting muscles, and bracing may provide joint stability and help minimize articular injury [4]. Elimination of brainstem deformation by straightening and stabilizing the craniocervical junction (via fusion surgery) may also improve pain, neural functioning, and quality of life [8].

Conclusion: A Paradigm Shift in the Etiology of Functional Somatic Syndromes

Disorders that lack “objective markers” are usually considered to be functional, not “organic.” This implies to some that the symptoms in functional somatic syndromes are physiological manifestations of psychosocial factors, a view that enforces an insular attitude to the etiology of disease rather than an interactive holistic approach. Consequently, when investigative results are negative, management is commonly limited to reassurance about the (apparent) absence of disease and occasionally psychiatric therapy. These treatments, however, are unpopular with patients, have low coherence rates, and seldom provide long-term therapeutic relief [41][43].

An alternative explanation is that the organic abnormalities are undetectable through cursory diagnostic testing as the underlying mechanism may be histopathological in origin, or, as seen in the case of upright MRIs on EDS-HT patients, the body may not be in the problematic position when testing takes place. The overly common cognitive error overshadowing high-tech medicine –that emotional issues are the underpinnings of illnesses lacking objectivity– must be overcome. While it is sufficient to say that, like virtually all known illnesses, psychosocial factors do play some role in functional somatic syndromes [1], an over emphasis on medically unexplained symptoms as being psychological bases causal reasoning on a negative. An absence of evidence does not denote an absence of organic disease –it simply means that the conditions that were tested for are not present in the individual and there is an infinite realm of alternative possibilities, such as EDS-HT.

Functional somatic disorders can only be successfully managed in the healthcare setting once a comprehensive understanding of their nature and treatment is acquired. The recognition of Ehlers-Danlos Syndrome Hypermobility type, and other disorders involving ligamentous laxity, as a possible physiological mechanism underlying various medically unexplained symptoms will help bridge the gap in physicians’ minds between described physical complaints and apparent negative test results in a subset of patients. Henceforth, in the wake of this disclosed correlation, further investigation into the role hypermobility and connective tissue abnormalities play in the etiology of these conditions, alongside a redefinition and modification of the diagnostic criteria of functional somatic syndromes, is essential to study of medically unexplained phenomena.

the above image is from Chronic Illness Cat and the below article is taken from EDS Info, a wonderfully informative blog for any chronic pain sufferer, which you should all go check out and bookmark and return to often.

UNTREATED CHRONIC PAIN IS ACUTE PAIN

The physiological changes associated with acute pain, and their intimate neurological relationship with brain centers controlling emotion, and the evolutionary purpose of these normal bodily responses, are classically understood as the “Fight or Flight” reaction,

When these adaptive physiologic responses outlive their usefulness the fight or flight response becomes pathological, leading to chronic cardiovascular stress, hyperglycemia which both predisposes to and worsens diabetes, splanchnic vasoconstriction leading to impaired digestive function and potentially to catastrophic consequences such as mesenteric insufficiency.

Unrelieved pain can be accurately thought of as the “universal complicator” which worsens all co-existing medical or psychiatric problems through the stress mechanisms reviewed above, and by inducing cognitive and behavioral changes in the sufferer that can interfere with obtaining needed medical care

Dr. Daniel Carr, director of the New England Medical Center, put it this way:

Chronic pain is like water damage to a house – if it goes on long enough, the house collapses,” [sighs Dr. Carr] “By the time most patients make their way to a pain clinic, it’s very late. What the majority of doctors see in a chronic-pain patient is an overwhelming, off-putting ruin: a ruined body and a ruined life.”

Dr. Carr is exactly right, and the relentless presence of pain has more than immediate effects. The duration of pain, especially when never interrupted by truly pain-free times, creates a cumulative impact on our lives.

CONSEQUENCES OF UNTREATED AND INADEQUATELY-TREATED PAIN

we must also consider often profound decrements in family and occupational functioning, and iatrogenic morbidity consequent to the very common mis-identification of pain patient as drug seeker.

The overall deleterious effect of chronic pain on an individual’s existence and outlook is so overwhelming that it cannot be overstated. The risk of death by suicide is more than doubled in chronic pain patients, relative to national rates.

What happens to patients denied needed pharmacological pain relief is well documented. For example, morbidity and mortality resulting from the high incidence of moderate to severe postoperative pain continues to be a major problem despite an array of available advanced analgesic technology

Patients who received less than 10 mg of parenteral morphine sulfate equivalents per day were more likely to develop delirium than patients who received more analgesia (RR 5.4, 95% CI 2.4–12.3)… Avoiding opioids or using very low doses of opioids increased the risk of delirium. Cognitively intact patients with undertreated pain were nine times more likely to develop delirium than patients whose pain was adequately treated. Undertreated pain and inadequate analgesia appear to be risk factors for delirium in frail older adults. [7]

PAIN SUFFERERS ARE MEDICALLY DISCRIMINATED AGAINST

Chronic pain patients are routinely treated as a special class of patient, often with severely restricted liberties – prevented from consulting multiple physicians and using multiple pharmacies as they might please, for example, and in many cases have little say in what treatment modalities or which medications will be used. These are basic liberties unquestioned in a free society for every other class of sufferer

chronic pain patients are often seen by medical professionals primarily as prescription or medication problems, rather than as whole individuals who very often present an array of complex comorbid medical, psychological, and social problems

Instead these complex general medical patients are ‘cared for’ as if their primary and only medical problem was taking prescribed analgesic medication.

This attitude explains why most so-called Pain Treatment Centers have reshaped themselves into Addiction Treatment Centers. Even with a documented cause for pain, the primary goal of these programs, whether stated or not, is to coerce patients to stop taking their pain medications.

This may work for a small number of pain patients who may not really need opioids in the first place, but is a “cruel and unusual” punishment for those of us with serious, documented, pain-causing illnesses.

The published success rate of these programs has nothing to do with pain – it is measured by how many people leave the program taking no pain medication, but there is no data about the aftermath, how many manage to stay off their medication long-term.

their obvious primary medical need is for medical stabilization, not knee-jerk detoxification

CHRONIC PAIN IS A LEGITIMATE MEDICAL DISEASE

Chronic pain is probably the most disabling, and most preventable, sequelae to untreated, and inadequately treated, severe pain.

Following a painful trauma or disease, chronicity of pain may develop in the absence of effective relief. A continuous flow of pain signals into the pain mediating pathways of the dorsal horn of the spinal cord alters those pathways through physiological processes known as central sensitization, and neuroplasticity. The end result is the disease of chronic pain in which a damaged nervous system becomes the pain source generator separated from whatever the initial pain source was.

Aggressive treatment of severe pain, capable of protecting these critical spinal pain tracts, is the standard care recommended in order to achieve satisfactory relief and prevention of intractable chronic pain

Medications represent the mainstay therapeutic approach to patients with acute or chronic pain syndromes… aimed at controlling the mechanisms of nociception, [the] complex biochemical activity [occurring] along and within the pain pathways of the peripheral and central nervous system (CNS)… Aggressive treatment of severe pain is recommended in order to achieve satisfactory relief and prevention of intractable chronic pain.

we are seeing ominous scientific evidence in modern imaging studies of a maladaptive and abnormal persistence of brain activity associated with loss of brain mass in the chronic pain population

Atrophy is most advanced in the areas of the brain that process pain and emotions. In a 2006 news article, a researcher into the pathophysiological effects of chronic pain on brain anatomy and cognitive/emotional functioning, explained:

This constant firing of neurons in these regions of the brain could cause permanent damage, Chialvo said. “We know when neurons fire too much they may change their connections with other neurons or even die because they can’t sustain high activity for so long,” he explained

It is well known that chronic pain can result in anxiety, depression and reduced quality of life

The areas involved include the prefrontal cortex and the thalamus, the part of the brain especially involved with cognition and emotions

The magnitude of this decrease is equivalent to the gray matter volume lost in 10–20 years of normal aging. The decreased volume was related to pain duration, indicating a 1.3 cm3 loss of gray matter for every year of chronic pain

clinicians have used opioid preparations to good analgesic effect since recorded history.

In fields of medicine involving controlled substances, especially addiction medicine and pain medicine, the doctor-patient relationship has become grossly distorted.

doctors-in-good-standing who, faced with a patient in pain and therefore at risk of triggering an investigation, modify their treatment in an attempt to avoid regulatory attention

Examples include a blanket refusal to prescribe controlled substances even when clearly indicated, or selecting less effective and more toxic non-controlled medications when a trial of opioid analgesics would be in the best interests of a particular patient. At the very least, some degree of suspicion and mistrust will surely arise in any medical relationship involving controlled substances.

the quality of care most physicians provide is fairly close to the medical standard of care which is what the textbooks say one should do, and which is generally in line with core medical ethical obligations

For example, modern pain management textbooks universally recommend ‘titration to effect’ (simplistically: gradually increasing the opioid dose until the pain is relieved or until untreatable side effects prevent further dosage increase) as the procedure by which one properly treats chronic pain with opioid medications. Yet the overwhelmingly physicians in America do not practice titration to effect, or anything even vaguely resembling it, for fear of becoming ‘high dose prescriber’ targets of federal or state law enforcement.

It is a foundation of medicine back to ancient times that a primary obligation of a physician is to relieve suffering. A physician also has a fiduciary duty to act in the best interests of the individual patient at all times, and that the interests of the patient are to be held above all others, including those of family or the state.[23] These ethical obligations incumbent on all individual physicians extend to state licensing and regulatory boards which are composed of physicians monitoring and regulating themselves. [24]

A number of barriers to effective pain relief have been identified and include:

The failure of clinicians to identify pain relief as a priority in patient care;

Fear of regulatory scrutiny of prescribing practices for opioid analgesics;

The persistence of irrational beliefs and unsubstantiated fears about addiction, tolerance, dependence, and adverse side effects of opioid analgesics.

A rift has developed between the usual custom and practice standard of care (the medical community norm – what most reputable physicians do) and the reasonable physician standard of care (what the textbooks say to do – the medical standard of care), and this raises very serious and difficult dilemma for both individual physicians and medical board

Research into pathophysiology and natural history of chronic pain have dramatically altered our understanding of what chronic pain is, what causes it, and the changes in spinal cord and brain structure and function that mediate the disease process of chronic pain, which is generally progressive and neurodegenerative

This understanding explains many clinical observations in chronic pain patients, such as phantom limb syndrome, that the pain spreads to new areas of the body not involved in the initiating injury, and that it generally worsens if not aggressively treated. The progressive, neurodegenerational nature of chronic pain was recently shown in several imaging studies showing significant losses of neocortical grey matter in the prefrontal lobes and thalamus

Regarding the standard of care for pain management:

1) Delaying aggressive opioid therapy in favor of trying everything else first is not rational based on a modern, scientific understanding of the pathophysiology of chronic pain, and is therefore not the standard of care. Delaying opioid therapy could result in the disease of chronic pain.

More reality checks when it comes to chronic pain and opiates, via a super smart fellow blogger! So happy to print this and put it in my medical binder for those idiots who think I should just suffer endlessly, needlessly, and be happy for the privilege.

It’s just so wonderful when people form an opinion based on facts and not histrionics.

TRUE: Opiates do not remove chronic pain, they do not numb pain like Novocain, they merely dull it enough so that it isn’t all-consuming.

2. false: Pain is the body trying to tell you to stop, so you shouldn’t take opiates to cover up the pain signals.

TRUE: Normal pain is an alarm to take action, but chronic pain happens when the alarm gets stuck in the “on” position – the switch itself is broken.

3. false: Opiates make you dull, confused, and non-functional.

TRUE: When used for pain relief, opiates allow people to be more active and functional, get out of the house and socialize, sometimes even continue working.

4. false: There are other pain medications that work just as well as opiates.

TRUE: Opiates are the most (and often the only) effective medications for pain.

5. false: Opiates have severe and permanently damaging side effects.

TRUE: Opiates have fewer and lesser side-effects than most of the other medications prescribed for pain.

6. false: You will get addicted if taking opiates.

TRUE: People taking opiates for pain are statistically unlikely to become addicted unless they already have addictive tendencies (5% chance). However, regular use of many medications causes dependence after your body has adjusted to them.

7. false: If you take opiates for too long, you’ll get hyperalgesia.

TRUE: Opiate-induced hyperalgesia is extremely rare in humans, and this scare tactic is based on just a handful of very small research studies.

8. false: If the pain is constant, you’ll get used to it and it won’t hurt as much.

TRUE: Pain that is allowed to persist uncontrolled leads to changes in the nerves that can eventually become permanent.

9. false: Opiates work the same way for everyone.

TRUE: Different people get the same amount of pain relief from widely varying dosages because our bodies are all different in the way we “digest” opiates.

10. false: It’s better not to take opiates because they damage the nervous system and cause hormonal imbalances.

TRUE: Persistent pain results in the same kind of damages to the nervous and hormonal systems.

11. false: You should not take opiates because your pain won’t improve.

TRUE: Chronic pain can only be treated, not cured. Opiates are often the best means available to treat the devastating pain symptoms until a cure is found.

12. false: If you start taking opiates, you’ll just have to take more and more forever.

TRUE: Most chronic pain patients finds a stable dose of opiates that works for them. If doses need to be increased, it is usually because the pain condition gets worse over time.

13. false: People only want opiates for the high.

TRUE: When taken as prescribed for chronic pain, opiates do not make you “high”. The same chemicals that make illegal users “high” go toward dulling the pain instead.

14. false: It’s better to tough it out.

TRUE: Denying people pain relief sentences them to a life of unnecessary suffering.

Many people disabled by chronic pain are unfairly accused of lying and faking, so here’s some myths from that category too:

1. false: People who complain about chronic pain are just trying to get SSDI.

TRUE: Most people disabled by pain desperately want to work. Many had to give up high-level, well-paying positions and now live in poverty on SSDI. There may some fakers, but this is not a reason to deny SSDI for truly disabling pain.

2. misleading: If injured workers are given opiates they are unlikely to return to work (statistically true)

TRUE: This is probably because their injuries are serious enough to cause chronic pain and require opiates, not because the opiates are keeping them away from work.

Printable Awareness Ribbon Chart

The website goes into much more detail, as well as noting extra conditions that are covered under each color/color combo. I’m thinking about doing all of the colors with each condition written on the ribbon in photoshop so that we can all save and post according ribbons if we want, without any confusion.

This search started, by the way, because a friend of mine from high school had posted a yellow ribbon as her facebook profile picture, and I wanted to know what cause she was representing, and the answer ranged from suicide awareness to bladder cancer to liver disease to supporting the troops. It wasn’t written on the ribbon, plus no clues were given via description, and that frustrates me. What’s the point if no one else knows what cause you are trying raise awareness for?

Anyhow, I will get around to that, but if illness interupts it could take some time. Hopefully I can make a resource page on this site where people can save a picture of the ribbon matching the condition(s) they have, to add to the bottom or sidebar of their page. And yes, when I do this, the first will be one for Chronic Pain all by itself, since many of us do not know why we are dealing with what we are dealing with, and many of will never know.

I might know a lot of you who fit into the “rare diseases” white ribbon, in which case, I want to make you a ribbon with your specific disease or condition or struggle, and you pick the color. Just drop me a comment, any time, either on this post or on the page I create later on. Post to come soon, hopefully with the first ten or so ribbons that are requested. I might do a poll on ribbon styling too…. it would be my first opportunity to use that option on WordPress!

Happy creative Saturday, friends ❤

I’m glad we all made it. This week felt like it just would not end, and I got next to nothing done. Hopefully this weekend I can shift gears, but I seem to be setting myself back with these big pushes when I get one slightly better day. Just so bored with this!!! I hate tv, so I’ve been trying to entertain myself other ways, and it’s hard! It’s only going to get harder when I ask my doctor for a heart rate monitor to keep track of my energy levels as best I can for a few months. Any time the damn thing beeps, I have to sit down. Doesn’t matter if I’m climbing the stairs, I gotta stop, sit down, record what time it is and what made it go off, and let my heart rate go back to a lower range. It sounds pretty frustrating, but who knows, it could provide me with some much-needed evidence-based data to share with my doctors, or it could provide me with just enough data to teach myself to live within my “energy envelope”. If you want to read more about heart rate monitoring and the logic behind it, check out these articles:

Like this:

It’s amazing how often health providers get asked directly or indirectly whether someone experiencing pain is ‘faking’ it. The short answer is the most accurate – we can’t tell. We’re not lie detectors, there is no ‘gold standard’ to work out whether someone is pretending or not, and the question is based on erroneous thinking about pain and pain behaviour.

I can almost feel the spluttering at my last sentence from some readers!

Let’s look at this more closely.

Remember the biopsychosocial model of pain states that the experience of pain and pain behaviour is influenced by three broad groups of factors: the biomedical/biophysical factors such as extent of tissue disruption at the periphery (or site of trauma), neurological changes of transmission and transduction (throughout the peripheral and central nervous system), and disturbance of the neuromatrix.

At the same time, there are psychological factors such as the level of…

Thank you for sharing!

Like this:

With a chronic illness or two (or five), it can feel, especially at first, like all mental growth and development is in limbo, that it is all so beyond you. Your ability to focus, care, be motivated/inspired, or be fully present in life is even suspended, and it takes a huge amount of effort to immerse yourself in any part of your day, from work to free time, pain can be so overriding that it even becomes difficult to focus on your significant other’s needs like you used to, or even to be aware of them. You feel terrible about these things, we all do. No one likes to have to compare our old selves to our new selves post chronic-illness.

And I respect that, very much so, and do not want to take away from the reality of those moments. Though I often try to emphasize the positive on this blog, I will be honest, there days when I have to write the positive message I want to say over and over again until I really believe it, or skip the “fake it till you make it” approach and wait to post something until I feel less cynical about life. I am often stumped by my own depression, my own guilt. My illnesses and pain often overwhelm me and leave me so hopeless I can’t even bear to write about it. I never want to trivialize the absolute difficulty of living in constant, unrelenting pain that threatens to burn you alive with its intensity. During times when I feel that terrible and dysfunctional in every way, I tend to shut down, pouring my energy into worry, fear of rejection, and often anger, among other negative emotions. I do not believe it is anyone’s “fault” if they feel upset about something. There is always, always, always a reason for why people feel and act the way they do, and though that may not excuse behavior that is harmful towards others, it also provides a framework for starting to understand those in all stages of recovery or maintenance with a chronic illness. Just because some of us happen to be really good at dealing with pain, and some of us do not handle it as well, does not make those of us who are struggling any less worthy of love or admiration for where we are in our life and what it has taken us to get there. It also does not give someone who is in a better place mentally, or who feels like they are in a better place, the right to demerit someone who is just starting out on this journey, or someone who is picking themselves up from the depths of hell for the 42nd time and trying again, or even someone who isn’t yet aware of the path in front of them and can only focus on their own misery all of the time. These are all stages of the same state of existing and trying to thrive with a chronic illness. We are no better or worse than anyone else in pain, or bedbound, or learning to walk again, or even than someone who has given up, spiralled deeper and deeper into the sadder side of illness. No one wants to suffer. We were not born aiming for misery. At no point did someone walk up to us and sell us this illness, we did not choose it, we would do anything to be better, and many of have done everything. This is hard. Bottom line. You are allowed to have days, weeks, months, years, where you feel like a failure. You are allowed to grieve, hurt, or be miserable. You are allowed to scream, cry, or feel the hollow, numb, hopeless apathy wash over you for a time. These are your emotions, you are supposed to feel both highs and lows, and all things in between.

No one gets to tell you that you aren’t dealing with your illness in the best possible way for you, even your doctor’s advice needs to be taken with a grain of salt and a deep knowledge of what is right for you, in a long-term sense. Listening to your intuition is confidence boosting, I promise. We are all doing our best, even if the whole world makes you feel like a scab on a wound stuck on the back of society, that is not our fault, and it is not forever! Nothing is worse than being stuck in the negative side of emotions, and on top of that, also feeling guilty for your own disordered thoughts.

Dear spoonies, you are doing the best you can. Please, please, try to take some comfort in the fact that there are people out there who know how hard you’re working, how every single day is a massive achievement, and how determined you really are underneath the tears, the desperation, and the bad habits that will not be dealt with right now.

You don’t have to think positive all the time. You can be loved anyway, no matter what side of the emotional spectrum you are currently leaning towards. You are still worth just as much when you are sad as when you are happy, so please don’t feel like just because you are depressed, you are worthless. Depression is a part of this. A study from 2008 at Northwestern University shows how pain actually changes our brains, and it takes some time to adjust to that change and figure out how to work around what you have been given. We are all different, there is no formula for everyone to achieve optimum happiness, and anyone that insists there is might not be as brilliant as they appear.

CHRONIC PAIN HARMS THE BRAIN

In a new study, investigators at the Feinberg School of Medicine have identified a clue that may explain how suffering long-term pain could trigger other pain-related symptoms.

Someone who tells you that it is possible to be chronically ill your whole life without dealing with bouts of depression, obviously hasn’t suffered any major trauma in their life, either that or they are in some deep denial. Whatever the reason for their skewed logic, don’t listen to that crap. Illness can be traumatizing, but you are safe in thinking your thoughts, no one has dominion over what you feel, you are in charge, you are allowed to experience the ups and the downs in life without censorship. Just in case you don’t have a safe place to be yourself in all of your disease’s ups and down, I am always honored to listen and encourage. No one should ever have to do this alone. Chronic illness is an adventure best enjoyed surrounded by those who understand and commend your quiet everyday courage just in getting up each morning to a body that does not behave and a life that is more stressful than most. I admire each and every one of you, even if we haven’t met yet, I know you’re trying and I’m rooting for you.

Beautiful spoonies, you all fight so hard, and that makes me so proud to be a part of this wonderful and supportive community. Even if we’re depressed from time to time, we are still fighting to be here in a meaningful way, and very much deserving of finding that. ❤

What to Do When You Have to Resort to the Emergency Room (When You Have a Chronic Illness)

A trip to the ER is no fun, no matter how you spin it. When you’re a chronic pain patient or someone with a chronic illness that can cause bouts of severe pain, it can be a complete and total nightmare.

A patient with chronic pain can help the Emergency Room staff to understand that their medical problems, especially pain, are a legitimate emergency by following a few guidelines and suggestions that will lessen some of the unpleasant drama of going to the ER.

Always bear in mind that the Emergency Room is a last resort, and Urgent Care will almost always turn away a patient with a chronic illness. Hospitals are so wrapped up in covering their asses legally that they have started turning away chronic pain patients much like Urgent Care does, even when the need for treatment is real and immediate.

Your regular healthcare team, especially your Primary Care Physician, is by far your best bet for getting help managing a chronic condition that is spiking out of control, but sometimes the ER is the only option. When that happens, here are some tips to help make your experience more manageable:

Make sure that you have a regular physician who treats your chronic pain. That’s a relationship that all chronic pain patients should establish before they ever set foot in an emergency room. Without this all-important steady doctor-patient relationship, the rest of this list is not really possible. In terms of seeking out aid in the Emergency Room for a spike or flare of pain having to do with an ongoing condition or problem, even having a bad doctor is better than no doctor at all. If you are having trouble finding a primary care physician who actually does care, the best place to start looking are local and even national support groups for your condition(s). They will have lists of hospitals and even specific doctors in your area who have been a good match for others in your situation. If those doctors are not taking patients, don’t be afraid to ask their staff where they would recommend going or if that doctor can make some recommendations of physicians they know to be effective at treating your condition. This search can take a while, but always keep a PCP on file, if you at all can. Not having a primary person who writes your prescriptions and handles your referrals makes the staff in an Emergency Room nervous no matter what.

Show that you have tried to contact your regular doctor before you go to the ER. If you have been in pain for five days and have not alerted your doctor, the ER staff will question how bad your pain really is. Even if the pain struck out of the blue that day, make an effort to contact your regular doctor first. ER staff will be more sympathetic to patients who have called their doctors and been told to go to the emergency room because the doctor was unable to see them. At least you’re showing you made an effort and only using the emergency room as your treatment of last resort, as opposed to the primary place you go for pain medication. This is important, as unfair as it is, they will not give you proper care if you are using the ER too liberally. Having your physician back up your story is never a bad thing, it helps establish legitimacy and urgency, and can help push you through to getting treatment sooner rather than making your wait for four hours “just to make sure you’re really in pain” before giving you any medication or imaging.

Bring a letter from your doctor. A letter from your physician, with a diagnosis and current treatment regimen, is a logical, completely reasonable thing to carry with you, particularly if you’re on a regular dose of opiates in today’s atmosphere of distrust and disbelief of pain patients. Always make sure the letter has your doctor’s name and phone number. That way, if ER doctors want to contact your physicians, they can. This is especially useful if you’re traveling or going to a hospital that you have never visited before.

Bring a list of medications. Bring a list of your medications, instead of relying on memory. Usually the hospital will already have access to the list of everything that you have taken for the past several years, so don’t try to lie about it, you will only hurt yourself in the long run. Always be honest about medications you have taken or have been prescribed.

Work cooperatively with emergency room staff. It might not be fair, but if a patient comes in screaming and shouting that they need pain medication right away, the staff isn’t going to like it. Being loud and distressed will call negative attention to your actions and makes hospital staff that much less sympathetic. You might be in agonizing pain, but the staff is going to be more concerned with “drug seeking behavior” than your well-being. So rather than demand things, try to work cooperatively with the staff, even if they’re not being cooperative with you.

If you have an alert card or pamphlet explaining your condition, hand it to them and ask for it to be put in your file. For instance, I keep a card in my wallet explaining that I have Ehlers-Danlos Syndrome and detailing the worst parts of the illness and information that is pertinent to an emergency. I also listed my most severe medical allergies around the border of the card in big black block letters. It’s important for the staff to know what is going to cause more pain & what may help. If you have a rare condition or one that is frequently misunderstood or which is conventionally thought to not cause pain, bring a relevant pamphlet from an awareness or advocacy group with you. Consider buying one of the brand new USB alert bracelets, pendants, or wallet cards. These plug into a computer in an emergency if you can’t speak for yourself, and they can be uploaded with as detailed medical information as you want, from medical history and current doctors with phone number and addresses to info like allergies, current medications, current medical concerns, and alternative treatments/supplements as well. Most manufacturers understand that a computer might not be nearby in an emergency and have a phone number printed on the back of the bracelet that you can call to access the information as well.

Ask for a nurse advocate or make sure someone is with you. This will help you when trying to explain things to the staff. It helps to have another person there to advocate for you.

If at all possible, use the same Emergency Department as the last one you went to, your pain will be that much more believable if you always use the same place. Plus, you might actually get doctors to take an interest in your chronic pain condition and maybe even other conditions that can cause a chronic illness patient to end up in the ER. Think about the ramifications that could have down the road for future patients!

Finally, since there are a lot of easy-to-forget details in this list, especially in the fog/panic/blacking out that happen whilst in horrific pain, I like to keep a folder handy with all those details written down, as well as a copy of most everything I need to bring with me. It isn’t always updated with the newest things I’m taking, so I bring the bottles themselves if I am on anything different since the last list was written. Being organized shows the ER team that not only do you take your condition(s) seriously, but that you have done all you possibly can to avoid the Emergency Room and to only use it as a very last resort.

About two years ago I was turned away from an ER without treatment by an extremely ignorant physician (after toughing it out all night crying and screaming at home), I had to contact and be seen by my pain doctor the next morning and then was sent right back to the same ER, only this time I was told to have them call my pain clinic when I got checked in. I did not want to go back there, but things went a lot smoother the second time, despite my apprehension. I was given the correct sedatives for once, and no one yelled at me or gave me super judgmental looks. I was treated for pain, monitored, and released without being asked to pee in a cup or otherwise treated like an addict. It was the only decent Emergency Room experience I have ever had, other than being in constant, black-out, vomiting, excruciating, unrelenting pain for almost 48 hours prior to finally receiving treatment and not sleeping a single hour of that time, all from an Occipital Nerve Block injection that was supposed to be a diagnostic tool, gone horribly wrong. (Hint: If your gut says “Do not do this, it isn’t safe” then listen to your gut, or it probably isn’t going to turn out well. I knew in my soul that the injection wasn’t going to be a good thing for me, and I don’t even have a minor fear of needles.)

I haven’t been back to the ER since, I have to admit I have stayed at home through even worse pain than that episode since then. No part of my soul trusts the Emergency Room to treat me, as a 26 year old fibromyalgia and chronic fatigue syndrome patient with occipital and trigeminal neuralgia, Spina Bifida Occulta, Joint Hypermobility Syndrome / Ehlers-Danlos Syndrome, with damaged vertebral discs at the top and bottom of my spine and everywhere in between, just to name a few of my chronic pain conditions. None of that matters because what they see is a 26 year old who usually hasn’t showered in days, is twitchy and fidgety (pain makes me move nonstop sometimes), sweating profusely (a fibro symptom having to do with autonomic nervous system dysfunction or dysautonomia), has a hard time walking in a straight line, and usually I am extremely angry or panicky, one or the other. There isn’t a lot of sympathy for me if I don’t do absolutely everything right when I go to the ER.

It’s certainly not fair or acceptable, but the Emergency Room just is not cut out for dealing with us; the tough cases, the ones who can’t be “fixed” or “stabilized” because in hospital staff eyes, we are already stabilized and not in any immediate danger. Yet pain is dangerous. Chronic pain over a life time is more damaging to the actual structures of the brain than taking hardcore opiates every single day for the rest of your life. That isn’t to say that there isn’t some middle ground to be found here. I do not advise hardcore opiates for chronic pain on a daily basis, especially at my young age, because if I take heavy opiates now, years down the road when I need surgery or dose titration, eventually there isn’t anything else to elevate to by the time I’m in my late 30’s. That’s not how I want to end up. The only painkillers I currently take are tramadol and medical marijuana for breakthrough pain and seizure activity.

I have learned to make do, but it took years to figure out just how little I can do off of painkillers without my condition deteriorating. I’m still trying to adjust, trying to find the sweet spot between too much and not enough, and knowing that life with a chronic illness will always be a delicate balancing act.

I’m not just saying that heavy opiates are a bad idea, I actually used to take anywhere from two to ten 5mg oxycodone every day while I was working, and while it did not take the pain away, it made me more able to do things, more willing to put myself through pain over and over again all day long. I could still drive at that time, purely thanks to opioid medications, but I don’t think it was healthy to push so hard that I had to pop pain pills like mints, just to stay upright and not cry through my whole shift. At one point I was even on morphine every night to sleep just a couple of hours. Clinics were offering me methadone, which I vehemently turned down.

I had a rough, rough few months when my first visit with a brand new doctor ended with him taking me off oxy and morphine all of a sudden in the middle of a flare, no weaning, just completely off of opiates (and chronic fatigue/ADHD medication at the same time too) cold turkey without even the slightest heads up. I wasn’t even given tramadol by that asshole. I had to call crying in pain four times in one week before he would even write a tiny prescription for 12 (yes, one freaking dozen) during a two month long flare up! So humiliating. He also told me not to go to the ER no matter how much pain I was in. What a complete idiot. His favorite phrase was “at least you’re not in a wheelchair”. I could barely contain my hatred every time he said that or my other personal favorite: “you are a perfectly healthy young woman”. Not my weight, my blood pressure, my mental health, or my chronic pain conditions were healthy about me, so I was partly just shocked he couldn’t think of any reasons I wasn’t healthy.

Yes, middle ground. I understand that “as-needed” for a chronic pain patient can mean literally anything, from almost never to pretty much always. So I say with caution and leaning more toward the almost never side of things, “as needed” pain medication can save your life. When you need it, and you really need it, you know best, and you deserve to be treated correctly, efficiently, and even compassionately by ER staff. The above suggestions should help cut down on the emotional trauma that people with chronic pain often associate with going to the Emergency Room. In our greatest time of need, it would be nice to actually be able to count on getting help when we seek out this last resort in our coping toolbox.

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Please help spread the word about malfunction of the autonomic nervous system and the many chronic conditions it can cause. There is no cure for dysautonomia, it is an invisible illness, and from day to day and person to person the symptoms vary widely as does the severity, so life with any of these illnesses is a roller-coaster, to say the least. Some people are just dizzy all the time and may not even realize that it isn’t normal, and severely affected individuals faint every time they stand or even sit up for a period of time, sometimes just a few seconds.

No one bothered to teach me about the autonomic nervous system. In a perfect world, doctors would explain these things to patients who are experiencing classic symptoms of ANS malfunction, as I am, and they would explain just how involved the ANS is in so many processes throughout the body. Normally, when you are in pain or experiencing stress, your autonomic nervous system ramps up your blood pressure, makes you sweat, and elevates your heart rate. When the pain or stressor is gone, your ANS should quiet right back down again, returning to baseline (and you with it). Yet, the ANS can become part of a long-term issue like chronic pain as well. It can become so agitated that it is constantly hyperactive; constantly on. This can exhaust your body and cause a cascade of other symptoms, some of which may be unexpected such as chronic fatigue, gastroparesis, severe migraines, adrenal fatigue, increased chronic pain, and immune issues.

First, it’s important to understand just how much the autonomic nervous system matters. It is involved in most involuntary actions in the body, such as heart rate, blood pressure, digestion, kidney function, and temperature control. Imagine never being the right temperature; always being too hot or too cold. When I say “too cold” I don’t mean in a mildly uncomfortable way, the way it feels to be out in the cold too long for a regular person, instead it can actually be quite painful and feel like it’s coming from the inside out, stinging and zapping your skin anywhere it touches articles of clothing or a draft hits it. Standing up with dysautonomia is like climbing Mt. Everest on bad days, and the constant headaches and digestive issues get old, fast.

Here is a pretty good overview of what Dysautonomia is, who gets it, what treatments are available, and other useful tidbits of info about ANS malfunction, so you can help spread awareness this month;

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